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CSF Leakage Syndrome
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Symptomatic leakage of CSF
            • Postoperative, iatrogenic, or post-traumatic CSF leak, ± pseudomeningocele

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Other Modality Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Kranz PG et al: Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance. AJNR Am J Neuroradiol. 34(6):1284-9, 2013
                                        2. Akbar JJ et al: The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                        3. Luetmer PH et al: When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 33(4):690-4, 2012
                                        4. Alaraj A et al: Angiographic features of "brain sag". J Neurosurg. 115(3):586-91, 2011
                                        5. Cho KI et al: Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 76(13):1139-44, 2011
                                        6. Kranz PG, Gray L, Taylor JN. CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 32(5):832-8, 2011
                                        7. Rahman M et al: Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery. 69(1):4-14, 2011
                                        8. Thomas DL et al: Radionuclide cisternography in detecting cerebrospinal fluid leak in spontaneous intracranial hypotension: a series of four case reports. Clin Nucl Med. 34(7):410-6, 2009
                                        9. Wang YF et al: Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 73(22):1892-8, 2009
                                        10. Albayram S et al: Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR Am J Neuroradiol. 29(1):116-21, 2008
                                        11. Tsai PH et al: Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension. Cephalalgia. 28(6):653-7, 2008
                                        12. Yoo HM et al: Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol. 29(4):649-54, 2008
                                        13. Tsai PH et al: Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study. Cephalalgia. 27(8):929-34, 2007
                                        14. Cohen-Gadol AA et al: Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery. 58(4 Suppl 2):ONS-238-45; discussion ONS-245, 2006
                                        15. Kelley GR et al: Sinking brain syndrome: craniotomy can precipitate brainstem herniation in CSF hypovolemia. Neurology. 62(1):157, 2004
                                        16. Eross EJ et al: Prolonged neurologic complication and MRI abnormalities consequent to intracranial hypotension. Headache. 43(4):415, 2003
                                        17. Luetmer PH et al: Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 24(8):1711-4, 2003
                                        18. Rothrock JF: Low pressure headache and pseudosubdural hematomas. Headache. 43(9):1009, 2003
                                        19. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leak associated with "nude nerve root" syndrome: case report. Neurosurgery. 53(5):1216-8; discussion 1218-9, 2003
                                        20. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. J Neurosurg. 99(5):840-2, 2003
                                        21. Whiteley W et al: Spontaneous intracranial hypotension causing confusion and coma: a headache for the neurologist and the neurosurgeon. Br J Neurosurg. 17(5):456-8, 2003
                                        22. Black P: Cerebrospinal fluid leaks following spinal surgery: use of fat grafts for prevention and repair. Technical note. J Neurosurg. 96(2 Suppl):250-2, 2002
                                        23. Chen CJ et al: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology. 44(12):996-1003, 2002
                                        24. Fujimaki H et al: Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension. Surg Neurol. 58(3-4):280-4; discussion 284-5, 2002
                                        25. Schrijver I et al: Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg. 96(3):483-9, 2002
                                        26. Dillon WP: Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol. 22(7):1233-4, 2001
                                        27. Yousry I et al: Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol. 22(7):1239-50, 2001
                                        28. Patel MR et al: CT-guided percutaneous fibrin glue therapy of cerebrospinal fluid leaks in the spine after surgery. AJR Am J Roentgenol. 175(2):443-6, 2000
                                        29. O'Carroll CP et al: The syndrome of spontaneous intracranial hypotension. Cephalalgia. 19(2):80-7, 1999
                                        30. Atkinson JL et al: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 88(2):237-42, 1998
                                        31. Moayeri NN et al: Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg. 88(5):912-8, 1998
                                        32. Rabin BM et al: Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol. 19(6):1034-9, 1998
                                        33. Vakharia SB et al: Magnetic resonance imaging of cerebrospinal fluid leak and tamponade effect of blood patch in postdural puncture headache. Anesth Analg. 84(3):585-90, 1997
                                        34. McCormack BM et al: Pseudomeningocele/CSF fistula in a patient with lumbar spinal implants treated with epidural blood patch and a brief course of closed subarachnoid drainage. A case report. Spine. 21(19):2273-6, 1996
                                        35. Renowden SA et al: Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 59(5):511-5, 1995
                                        36. Maycock NF et al: Post-laminectomy cerebrospinal fluid fistula treated with epidural blood patch. Spine. 19(19):2223-5, 1994
                                        37. Lee KS et al: Postlaminectomy lumbar pseudomeningocele: report of four cases. Neurosurgery. 30(1):111-4, 1992
                                        38. Waisman M et al: Postoperative cerebrospinal fluid leakage after lumbar spine operations. Conservative treatment. Spine. 16(1):52-3, 1991
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Definitions

                                                  • Symptomatic leakage of CSF
                                                  • Postoperative, iatrogenic, or post-traumatic CSF leak, ± pseudomeningocele

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Other Modality Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Kranz PG et al: Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance. AJNR Am J Neuroradiol. 34(6):1284-9, 2013
                                                                              2. Akbar JJ et al: The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                                                              3. Luetmer PH et al: When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 33(4):690-4, 2012
                                                                              4. Alaraj A et al: Angiographic features of "brain sag". J Neurosurg. 115(3):586-91, 2011
                                                                              5. Cho KI et al: Spontaneous intracranial hypotension: efficacy of radiologic targeting vs blind blood patch. Neurology. 76(13):1139-44, 2011
                                                                              6. Kranz PG, Gray L, Taylor JN. CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension. AJNR Am J Neuroradiol. 32(5):832-8, 2011
                                                                              7. Rahman M et al: Spontaneous intracranial hypotension: dilemmas in diagnosis. Neurosurgery. 69(1):4-14, 2011
                                                                              8. Thomas DL et al: Radionuclide cisternography in detecting cerebrospinal fluid leak in spontaneous intracranial hypotension: a series of four case reports. Clin Nucl Med. 34(7):410-6, 2009
                                                                              9. Wang YF et al: Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology. 73(22):1892-8, 2009
                                                                              10. Albayram S et al: Gadolinium-enhanced MR cisternography to evaluate dural leaks in intracranial hypotension syndrome. AJNR Am J Neuroradiol. 29(1):116-21, 2008
                                                                              11. Tsai PH et al: Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension. Cephalalgia. 28(6):653-7, 2008
                                                                              12. Yoo HM et al: Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol. 29(4):649-54, 2008
                                                                              13. Tsai PH et al: Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study. Cephalalgia. 27(8):929-34, 2007
                                                                              14. Cohen-Gadol AA et al: Surgical anatomy of dural defects in spontaneous spinal cerebrospinal fluid leaks. Neurosurgery. 58(4 Suppl 2):ONS-238-45; discussion ONS-245, 2006
                                                                              15. Kelley GR et al: Sinking brain syndrome: craniotomy can precipitate brainstem herniation in CSF hypovolemia. Neurology. 62(1):157, 2004
                                                                              16. Eross EJ et al: Prolonged neurologic complication and MRI abnormalities consequent to intracranial hypotension. Headache. 43(4):415, 2003
                                                                              17. Luetmer PH et al: Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks. AJNR Am J Neuroradiol. 24(8):1711-4, 2003
                                                                              18. Rothrock JF: Low pressure headache and pseudosubdural hematomas. Headache. 43(9):1009, 2003
                                                                              19. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leak associated with "nude nerve root" syndrome: case report. Neurosurgery. 53(5):1216-8; discussion 1218-9, 2003
                                                                              20. Schievink WI et al: Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. J Neurosurg. 99(5):840-2, 2003
                                                                              21. Whiteley W et al: Spontaneous intracranial hypotension causing confusion and coma: a headache for the neurologist and the neurosurgeon. Br J Neurosurg. 17(5):456-8, 2003
                                                                              22. Black P: Cerebrospinal fluid leaks following spinal surgery: use of fat grafts for prevention and repair. Technical note. J Neurosurg. 96(2 Suppl):250-2, 2002
                                                                              23. Chen CJ et al: Spinal MR findings in spontaneous intracranial hypotension. Neuroradiology. 44(12):996-1003, 2002
                                                                              24. Fujimaki H et al: Cerebrospinal fluid leak demonstrated by three-dimensional computed tomographic myelography in patients with spontaneous intracranial hypotension. Surg Neurol. 58(3-4):280-4; discussion 284-5, 2002
                                                                              25. Schrijver I et al: Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy. J Neurosurg. 96(3):483-9, 2002
                                                                              26. Dillon WP: Spinal manifestations of intracranial hypotension. AJNR Am J Neuroradiol. 22(7):1233-4, 2001
                                                                              27. Yousry I et al: Cervical MR imaging in postural headache: MR signs and pathophysiological implications. AJNR Am J Neuroradiol. 22(7):1239-50, 2001
                                                                              28. Patel MR et al: CT-guided percutaneous fibrin glue therapy of cerebrospinal fluid leaks in the spine after surgery. AJR Am J Roentgenol. 175(2):443-6, 2000
                                                                              29. O'Carroll CP et al: The syndrome of spontaneous intracranial hypotension. Cephalalgia. 19(2):80-7, 1999
                                                                              30. Atkinson JL et al: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg. 88(2):237-42, 1998
                                                                              31. Moayeri NN et al: Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. Report of three cases and review of the literature. J Neurosurg. 88(5):912-8, 1998
                                                                              32. Rabin BM et al: Spontaneous intracranial hypotension: spinal MR findings. AJNR Am J Neuroradiol. 19(6):1034-9, 1998
                                                                              33. Vakharia SB et al: Magnetic resonance imaging of cerebrospinal fluid leak and tamponade effect of blood patch in postdural puncture headache. Anesth Analg. 84(3):585-90, 1997
                                                                              34. McCormack BM et al: Pseudomeningocele/CSF fistula in a patient with lumbar spinal implants treated with epidural blood patch and a brief course of closed subarachnoid drainage. A case report. Spine. 21(19):2273-6, 1996
                                                                              35. Renowden SA et al: Spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry. 59(5):511-5, 1995
                                                                              36. Maycock NF et al: Post-laminectomy cerebrospinal fluid fistula treated with epidural blood patch. Spine. 19(19):2223-5, 1994
                                                                              37. Lee KS et al: Postlaminectomy lumbar pseudomeningocele: report of four cases. Neurosurgery. 30(1):111-4, 1992
                                                                              38. Waisman M et al: Postoperative cerebrospinal fluid leakage after lumbar spine operations. Conservative treatment. Spine. 16(1):52-3, 1991